A biological classification of Huntington's disease: the Integrated Staging System

SJ Tabrizi, S Schobel, EC Gantman… - The Lancet …, 2022 - thelancet.com
The current research paradigm for Huntington's disease is based on participants with overt
clinical phenotypes and does not address its pathophysiology nor the biomarker changes …

Potential disease-modifying therapies for Huntington's disease: lessons learned and future opportunities

SJ Tabrizi, C Estevez-Fraga… - The Lancet …, 2022 - thelancet.com
Huntington's disease is the most frequent autosomal dominant neurodegenerative disorder;
however, no disease-modifying interventions are available for patients with this disease. The …

Huntington's disease: a clinical review

P McColgan, SJ Tabrizi - European journal of neurology, 2018 - Wiley Online Library
Huntington's disease (HD) is a fully penetrant neurodegenerative disease caused by a
dominantly inherited CAG trinucleotide repeat expansion in the huntingtin gene on …

[HTML][HTML] Huntingtin lowering strategies for disease modification in Huntington's disease

SJ Tabrizi, R Ghosh, BR Leavitt - Neuron, 2019 - cell.com
Huntington's disease is caused by an abnormally expanded CAG repeat expansion in the
HTT gene, which confers a predominant toxic gain of function in the mutant huntingtin …

Diagnostic contribution and therapeutic perspectives of transcranial magnetic stimulation in dementia

V Di Lazzaro, R Bella, A Benussi, M Bologna… - Clinical …, 2021 - Elsevier
Transcranial magnetic stimulation (TMS) is a powerful tool to probe in vivo brain circuits, as it
allows to assess several cortical properties such as excitability, plasticity and connectivity in …

Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis

LM Byrne, FB Rodrigues, K Blennow, A Durr… - The Lancet …, 2017 - thelancet.com
Background Blood biomarkers of neuronal damage could facilitate clinical management of
and therapeutic development for Huntington's disease. We investigated whether …

Huntington's disease: mechanisms of pathogenesis and therapeutic strategies

M Jimenez-Sanchez, F Licitra… - Cold Spring …, 2017 - perspectivesinmedicine.cshlp.org
Huntington's disease is a late-onset neurodegenerative disease caused by a CAG
trinucleotide repeat in the gene encoding the huntingtin protein. Despite its well-defined …

Polyglutamine repeats in neurodegenerative diseases

AP Lieberman, VG Shakkottai… - Annual Review of …, 2019 - annualreviews.org
Among the age-dependent protein aggregation disorders, nine neurodegenerative diseases
are caused by expansions of CAG repeats encoding polyglutamine (polyQ) tracts. We …

Apathy in Parkinson's disease: clinical features, neural substrates, diagnosis, and treatment

J Pagonabarraga, J Kulisevsky, AP Strafella… - The Lancet …, 2015 - thelancet.com
Normal maintenance of human motivation depends on the integrity of subcortical structures
that link the prefrontal cortex with the limbic system. Structural and functional disruption of …

Therapies targeting DNA and RNA in Huntington's disease

EJ Wild, SJ Tabrizi - The Lancet Neurology, 2017 - thelancet.com
No disease-slowing treatment exists for Huntington's disease, but its monogenic inheritance
makes it an appealing candidate for the development of therapies targeting processes close …